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Idiopathic Entity
TM: Incidence
Rare: Estimated between 1 and 8 cases per million people per year 1400 new cases reported in US each year Affects individuals of all ages with a bimodal peak between ages 10-19 and 30-39
Presentation
50% will lose all movement in legs Nearly all have some degree of bladder dysfunction 80-94% have numbness, paresthesias, or band-like dysethesias Autonomic symptoms may include: urgency, incontinence, difficulty or inability to void, incomplete evacuation of bowel and/or bladder, sexual dysfunction 80% of patients reach clinical nadir within 10 days of symptom onset Thoracic spinal cord most typically involved in adults, cervical spinal cord in children
TM Diagnostic Criteria
Etiology
Acquired alteration in the innate or acquired immune system Cellular injury and dysfunction Infectious trigger: infectious agent triggers breakdown of immune tolerance for self-antigens TM and ADEM: Superantigen-mediated activation of T lymphocytes Suspected that multiple immune system components contribute to observed dysfunction including T and B lymphocytes, macrophages, and NK cells Mechanism of injury also probably involves multiple pathways including T lymphocyte killing of neural cells, cytokine injury, activation of toxic microglial pathways, immune-complex deposition, and apoptosis
TM and MS
TM can be the presenting feature of MS Patients ultimately diagnosed with MS are more likely to have:
asymmetric clinical findings predominant sensory symptoms with relative motor sparing MRI findings extending over fewer than two spinal segments abnormal brain MRI oligoclonal bands
Pathology
Treatment
No consensus guidelines Mainstays include:
corticosteroids: no randomized trials plasmapheresis: moderate to severe cases, or those who do not respond to steroids after 3-5 days Pulse dose IV cyclophosphamide CSF filtration therapy: spinal fluid is filtered for inflammatory factors (not available in US)
For severe, refractory cases: 2 year course of azothioprine, methotrexate, mycophenolate, or oral cyclophosphamide
Prognosis
Most will have monophasic disease Up to 20% will have recurrent inflammatory episodes within the spinal cord Significant recovery is unlikely if no improvement by 3 months
Full recovery Severe permanent disability
Recurrence
Predictors of recurrence:
Multifocal lesions within the spinal cord Demyelinating brain lesions CSF oligoclonal bands Mixed connective tissue disorder SS-A antibodies Persistently high IL-6 levels in CSF: thought to lead to high NO production and subsequent neural injury Initial complaint of back pain Rapid progression to maximal symptoms within hours of onset Spinal shock 14-3-3 protein, a marker of neuronal injury, in CSF during acute phase